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  • 5/24/2018 Anemia Cases

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    ANEMIA CASES

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    1. A 70-year-old woman presented with progressive weakness and fatigue.

    The symptoms had begun about a month earlier, and she no longer feltwell enough to do her housework or take her daily walk. Although herbreathing was normal at rest, she was too short of breath to walk morethan two or three blocks.

    The results of the complete blood cell count (CBC) performed in herphysician's office were:

    hemoglobin, 5.4 gm/dL;

    mean corpuscular volume (MCV), 103 m3;

    red cell distribution width (RDW), 19.8% (normal, 12%-15%),

    white blood cell count, 3,900/mm3 (48% neutrophils, 43% lymphocytes,8% monocytes, 1% eosinophils);

    and platelets, 62,000/mm3.

    Based on these results, the patient was hospitalized.

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    She had no history of recent bleeding, jaundice, fever, anemia, or heart

    disease. She had not been exposed to medications (other than occasional

    vitamins and aspirin) or toxins. She had not abused alcohol and had no

    previous hospitalizations. Findings on the physical examination wereunremarkable except for mild tachycardia at rest (96 bpm), a blood

    pressure of 146/84 mm Hg recumbent and 142/78 mm Hg standing, pallor,

    external hemorrhoids, and trace pitting edema of the feet. Neither the

    liver nor spleen were palpable. The stool was negative for occult blood.

    A chest x-ray was normal, and an electrocardiogram showed only sinus

    tachycardia. The blood urea nitrogen (BUN) level was 15 mg/dL; glucose,

    108 mg/dL; and total bilirubin, 1.2 mg/dL (normal,

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    A 57-year-old woman presents to the clinic for evaluation ofataxia, weakness. The patient has been taking a multivitaminpreparation.

    Hematocrit is 38%

    white blood cell count 4,000; platelet count 100,000

    What tests would you order next ?

    What are th?

    2.

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    3. A 39-year-old woman was referred to our institution for evaluation of anemia.

    She was known to have multiple comorbidities and had a baseline hemoglobinconcentration of approximately 10.5 g/dL. About 6 months before her referral,the patient began having recurrent episodes of severe anemia, withhemoglobin values as low as 3.5 g/dL. She had become transfusion-dependentand had received about 30 units of packed red blood cells (RBCs) in thepreceding 3 months. The patient denied any history of easy bruisability,menorrhagia, or overt evidence of bleeding from any site. Additionally, she

    denied any change in the appearance or color of her urine and had no historyof jaundice. There was no family history of anemia or any other hematologicdisorder. As an outpatient, she had undergone an extensive evaluation atanother institution, but results failed to provide an explanation for heranemia.

    The patient's medical history was remarkable for severe asthma, thought to be

    due to Churg-Strauss syndrome. She had a tunneled central venous catheterfor self-administration of intravenous corticosteroids at the earliest sign of anasthmatic exacerbation. Her other medications included bronchodilators,weekly erythropoietin injections, intravenous iron therapy, an antidepressant,and an anxiolytic.

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    At presentation, the patient's vital signs were normal. Physical

    examination was unremarkable except for mild generalized pallor. A

    complete blood count on the day of admission revealed the following

    (reference ranges shown parenthetically): hemoglobin, 4.9 g/dL (12.0-15.5

    g/dL); mean corpuscular volume (MCV), 94.4 fL (81.6-98.3 fL); hematocrit,

    13.4% (34.9%-44.5%); leukocyte count, 6.0 109/L (3.5-10.5 109/L); and

    platelet count, 203 109/L (150-450 109/L). The patient's partial

    thromboplastin time and prothrombin time (PT)/international normalized

    ratio were normal. These results were obtained within 24 hours of her last

    transfusion.

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    Which one of the following is the least likely in the differential diagnosis of

    this patient's anemia?

    Chronic blood loss

    Acute hemolysis

    Chronic disease Myelodysplastic syndrome

    Acquired pure red cell aplasia

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    With the observation that the patient's anemia was normocytic with an

    MCV of 94.4 fL, the next task was to narrow the list of differential

    diagnoses and establish whether this was due to premature destruction or

    acute loss of RBCs vs decreased bone marrow production.

    Which one of the following would be the next best test to narrow the list of

    differential diagnoses?

    Peripheral blood smear

    Absolute reticulocyte count

    Serum ferritin

    Erythropoietin

    Bone marrow biopsy and aspiration

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    The reticulocyte count is a good indicator of this and is the only test listed

    that could have directly provided this necessary piece of information.

    Our patient had a reticulocytosis of 13.3% (0.60%-1.83%), with an

    absolute reticulocyte count of 238.8 109/L (29.5-87.3 109/L).

    At this time, which one of the following series of tests would be most

    helpful in further narrowing the differential diagnosis?

    Total and indirect bilirubin levels, haptoglobin, lactate dehydrogenase

    (LDH)

    Peripheral blood smear

    Direct Coombs test

    Indirect Coombs test

    Activated partial thromboplastin time (aPTT), PT, fibrinogen, soluble fibrin

    monomer complex, and D-dimers

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    In this patient with an absolute reticulocytosis, ie, an adequate bone marrow response,the next step would be in differentiating between hemolysis and acute blood loss.

    Hemolysis is usually characterized by elevated indirect bilirubin concentrations,decreased serum haptoglobin concentrations (with intravascular hemolysis inparticular), and increased serum LDH levels, and this series of tests would be mostuseful in narrowing the differential diagnoses at this point.

    The peripheral blood smear is less specific, but in the presence of hemolysis, it mayreveal abnormally shaped RBCs, including fragmented RBCs (schistocytes, helmet cells),spherocytes, elliptocytes, or RBC inclusions, which may be seen in certain hemolysis-

    producing infections, such as malaria, babesiosis, and Bartonella.

    Hemolytic anemias may be acquired and immune, in which case there is immunologicdestruction of RBCs mediated by autoantibodies directed against antigens on thepatient's RBCs.

    The direct and indirect Coombs tests detect antibodies on the surface of the patient'sRBCs and in the patient's serum, respectively. However, the presence of hemolysis mustfirst be established, especially since a patient may have a mildly positive Coombs testthat is clinically insignificant if not associated with ongoing hemolysis.

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    The patient had a mildly reduced haptoglobin level at 14 mg/dL (30-200mg/dL), likely secondary to her multiple transfusions. However, her LDHlevel was normal at 205 U/L (122-222 U/L), as were her total and direct

    bilirubin levels at 0.4 mg/dL (0.1-1.0 mg/dL) and 0.1 mg/dL (0.0-0.3mg/dL), respectively.

    A peripheral blood smear showed no abnormally shaped RBCs. The overallpicture was not in keeping with hemolysis. On the first day of her

    evaluation, the patient's hemoglobin concentration was 11.1 g/dL.

    By day 2 of her outpatient work-up, it had decreased to 5.6 g/dL, and shereceived 4 units of packed RBCs.

    Despite the transfusions, her hemoglobin concentration decreased furtherwithin 24 hours to 4.9 g/dL. At this point, the patient was admitted andreceived 3 more units of packed RBCs. During this time, she wasasymptomatic, and her vital signs remained stable.

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    At this point, which one of the following would be the best step in the

    management of this patient?

    Esophagogastroduodenoscopy

    Colonoscopy

    Computed tomography (CT) of the abdomen and pelvis

    Transfer to the intensive care unit

    Angiography of the gastrointestinal (GI) tract

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    The patient had no overt signs or symptoms of bleeding, and it would be unlikely forher to have occult GI bleeding that resulted in such dramatic decreases in herhemoglobin concentration.

    Also, results of fecal occult blood testing were negative. Therefore, neither upper norlower GI endoscopy would be expected to reveal any useful information.

    However, the patient could have occult intra-abdominal bleeding, and noncontrast CTof her abdomen and pelvis would be crucial in ruling this out.

    The patient's mental status remained normal, and she was exhibiting no overt evidenceof decreased perfusion or hemodynamic instability other than mild tachycardia.

    Therefore, she could be deemed clinically stable, and transferring her to the intensivecare unit would be unnecessary at this time.

    She was well compensated despite the severity and acuteness of the anemia, no doubtin part due to her age and lack of other cardiac comorbidities. In this patient who isexhibiting no overt evidence of GI bleeding, angiography would not be the next beststep.

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    Noncontrast CT of her abdomen and pelvis revealed normal findings.During the night of hospital day 2, an astute nurse noticed what appearedto be bloodstains on the patient's gown.

    The patient reported that she had spilled cranberry juice on the gown.Closer inspection of her room revealed several blood-soaked tissues andStyrofoam cups filled with fresh blood in her wastebasket.

    The patient was also found to have dried, crusted blood all over her

    fingernails, and a blood-stained 10-mL syringe, most of its labeling wornaway by overuse, was found in her gown pocket

    Which one of the following is the most likely cause of this patient's anemia?

    Factitious disorder

    Munchausen by proxy

    Somatization disorder

    Hypochondriasis

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    With the discovery made in the patient's room, in particular the syringe, the patient'sself-phlebotomy became evident, leading to a diagnosis of factitious anemia.

    The most chronic and extreme form of factitious illness, Munchausen syndrome,typically includes travel from hospital to hospital combined with the willingness tosubmit to multiple procedures for self-fabricated signs of illness, as occurred with ourpatient before her presentation at our institution.

    In Munchausen by proxy, caregivers (usually mothers) induce illness in their children toobtain care and support for themselves.

    Somatization refers to the tendency to experience psychological distress in the form of

    somatic symptoms not intentionally produced, thus differentiating this disorder fromfactitious illness or malingering.

    Hypochondriasis refers to a preoccupation with believing one is ill as a result ofmisconstruing physical symptoms that are not self-generated.

    The patient was seen by the psychiatry service, and although she was obviously at risk

    of purposeful self-harm, she denied suicidal or homicidal ideation.

    It became evident that she had a history of severe depression, borderline personalitydisorder, chemical dependency, and a history of repeated episodes of parasuicide bymeans of wrist cutting.

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    4.

    a 33-year-old, well-conditioned male athlete who presented to a referring

    hospital with a 4-day history of fatigue, lethargy, fever, chills, jaundice,

    dark urine, and abdominal pain.

    His medical history included ocular toxoplasmosis in childhood. Several

    years before admission, he had become acutely anemic and was

    successfully treated with red blood cell (RBC) transfusions andimmunosuppression.

    He was then lost to follow-up until the onset of this illness. He took

    vitamins and nutritional supplements, used smokeless tobacco, and

    denied alcohol or drug abuse.

    He had recently started a job working with refrigerated and frozen food.

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    Sodium: 134 mEq/L (136-142)Potassium: 4 mEq/L (3.8-5.0)

    Chlorine: 104 mEq/L (95-103)

    Carbon dioxide: 22 mmol/L (24-30)

    Glucose: 172 mg/dL (70-110)

    Creatinine: 1.4 mg/dL (0.6-1.2)

    Blood urea nitrogen: 39 mg/dL (8-23)

    Calcium: 8.4 mg/dL (9.2-11.0)

    Magnesium: 1.9 mg/dL (1.8-3.0)Serum iron: 267 g/dL (60-150)

    Total iron-binding capacity: 291 g/dL (250-

    400)

    Transferrin: 208 mg/dL (215-380)

    Sedimentation rate: 119 mm/h (10-12)

    Acute hepatitis panel: negative

    White blood cell count: 16,200 cells/L

    Hemoglobin: 3.6 g/dL

    Hematocrit: 10.6%

    Mean corpuscular volume: 104.7 fL

    Platelet count: 340,000 cells/L

    Reticulocyte count: 8.4% (0.5%-2.3%)

    Haptoglobin: 6 mg/dL (36-195)

    Lactate dehydrogenase (LDH): 943 U/L(91-180)

    Aspartate aminotransferase: 100 U/L

    (8-33) Alanine aminotransferase: 35 U/L (4-36)

    Total bilirubin: 7.0 mg/dL (0.1-1.0)

    Direct bilirubin: 0.9 mg/dL (< 0.3)

    Albumin: 3.4 g/dL (3.2-4.5)

    Total protein: 7.0 g/dL (6.0-7.8)

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    Upon admission to our hospital, the patient's hemoglobin level was 4.6

    g/dL, and all other laboratory results were consistent with those from the

    referring hospital.

    Blood and urine cultures were negative.

    Peripheral smears demonstrated RBC agglutinates, microspherocytes,

    polychromasia, and nucleated RBCs.

    The patient had mixed-type warm IgG/IgM-mediated AIHA.

    The combination of massive intravascular hemolysis, vasoconstriction, and

    vaso-occlusion caused prolonged inadequate tissue oxygenation leading

    inexorably to multiple organ failure and death.

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    5.

    A 61-year-old woman was admitted to this hospital because of epigastric pain,vomiting, diarrhea, anemia, and acute kidney injury.

    The patient had been well until approximately 3 weeks before admission, whenvomiting, diarrhea, fevers, arthralgias, and episodes of epigastric pain of increasingfrequency and severity developed, which she attributed to a viral gastroenteritis.

    Two weeks before admission, epigastric and midabdominal pain worsened, with

    diarrhea and one episode of vomiting.

    The next day, she came to the emergency department at this hospital. She rated thepain at 4 on a scale of 0 to 10 (with 10 indicating the most severe pain) and reportedthat it was worse when she was lying flat.

    The blood pressure was 150/82 mm Hg, and the pulse 101 beats per minute; other vitalsigns were normal.

    The abdomen was soft, and there was mild epigastric tenderness without rebound; theremainder of the examination was normal.

    A stool specimen revealed occult blood.

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    Laboratory Data.

    Bazari H et al. N Engl J Med 2014;370:362-373.

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    Pertinent Clinical Details

    This 61-year-old woman presented with a 4-week history of epigastric pain, diarrhea,and vomiting.

    Arthralgias, fever, anemia, and acute kidney injury developed.

    The stool was guaiac-positive and positive for H. pylori antigen, and anesophagogastroduodenoscopy was normal.

    In the past, she had had hyperlipidemia and gastroesophageal reflux disease.

    On examination, she had mild abdominal tenderness.

    Pertinent laboratory values include progressive anemia, an absence of leukocytosis,progressive renal failure, elevated blood levels of aminotransferase and alkalinephosphatase, serum immune electrophoresis with no monoclonal protein detected, anda high serum free light-chain ratio (kappa:lambda ratio, 3.1; normal range, 0.3 to 1.7).

    Urinalysis was pathognomonic for an acute glomerulonephritis, with proteinuria andred-cell casts. The rheumatoid factor was weakly positive. Testing for ANA was negative,and blood levels of complement were low (C4, very low; and C3, slightly decreased).Evaluation for anemia was consistent with anemia of chronic disease. A bone marrowbiopsy specimen showed 3% monoclonal B cells, which were CD5 CD10 kappa+.

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    Serum free light-chain assays are the most sensitive tests for the detection ofabnormal immunoglobulin-secreting B-cell clones.

    Furthermore, flow-cytometric analysis of the bone marrow specimen confirmsthe presence of a small, clonal B-cell population, without an excess of plasmacells.

    The patient does not meet the criteria for a diagnosis of overt myeloma orlymphoma. It is possible that she has monoclonal gammopathy ofundetermined significance and a monoclonal B lymphocytosis both ofwhich are relatively frequent findings in older adults which may beunrelated to her current illness.

    Other considerations are cryoglobulinemia, immunotactoidglomerulonephritis, and deposition disease with light chains, light and heavychains, or heavy chains

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    6.

    A 22 month old boy presents to your office with a chief complaint of pallor.

    A visiting relative who has not seen the child for 5 months told his mother thatthe boy appears pale.

    The mother brings him in for a checkup even though she notices no change inhis coloring (he has always been fair skinned).

    On review of symptoms you find that he is an active toddler, with no recentfatigue, exercise intolerance, or increase in sleeping.

    He has had no blood in his diapers and no black or tarry stools.

    He is a picky eater, taking small amounts of chicken, pork and some

    vegetables, but loves milk and drinks six to eight bottles of whole milk per day.

    Family history reveals a distant aunt who had anemia when she was pregnantbut which subsequently resolved. There is no history of splenectomy, gallstones at an early age, or other anemia in the family.

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    Exam: VS: T 37.5, BP 90/52, P 145, RR 16, Height 85.5 cm (50th %ile), Weight

    13.2 kg (75th %ile). General appearance: He is a pale appearing, active toddler,

    holding a bottle, tearing and eating paper from your exam table. Eyes: No

    scleral icterus. Pale conjunctiva. Mouth: Dental caries. Chest: Clear. Heart:

    Mild tachycardia as above, grade II/VI systolic ejection murmur heard best

    over the upper left sternal border. Abdomen: No hepatosplenomegaly. Rectal:

    Dark brown, soft stool, negative for occult blood.

    CBC: WBC 6,100, Hgb 6.2 g/dl, Hct 19.8%, Plt 589,000, MCV 54 fL, RDW 17%.

    Reticulocyte count is 1.8%. The lab reports microcytosis, hypochromia, mild

    anisocytosis and polychromasia. There is no basophilic stippling.

    You correctly diagnose iron deficiency anemia, start oral iron and limit his milk

    intake. You see him in 3 days to assure compliance and his RDW is 27% and his

    reticulocyte count 17%. When you see him back in two weeks his mother is

    amazed at his new interest in table foods. His Hgb is now 8.5 g/dl, and his MCV

    64 fL. Two months later his hemoglobin has completely normalized, and you

    continue iron therapy for three more months.

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    7.

    ML is a 64-year old male who has not had any primary care for several

    years. When he tried to give blood last week, he was told that he was

    anemic. He presents to your clinic for evaluation.

    What would you do??

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    HPI: Ive been a little more tired than usual, but Ive been busy at work.

    Im getting close to retirement. Nothing else is unusual. I avoid doctors if I

    can

    PMH: Inguinal hernia repair 20 yrs ago FH: F & MGF-heart attack(age 80), brother-alcoholism

    SH: Married x44yr, smokes 1ppd, a couple beers/night

    MEDS: daily multivitamin

    ALLERGIES: none

    ROS:+fatigue, +urine seems a little darker lately

    Only a CBC w/ diff was obtained:

    WBC: 8.2, HCT 32.2, MCV 79, Platelets 221, differential - normal

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    Initial Thoughts?

    Blood loss?

    Age places him at risk for colon CA

    Decreased Production?

    Alcohol use, Iron deficiency

    Increased Destruction?

    Darker urine lately

    Peripheral Blood Smear Reticulocyte count Iron Studies

    Ferritin

    TIBC % Saturation

    Urinalysis Colonoscopy referal

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    More Results

    Smear reveals microcytic, microchromic RBCs

    Retic count is interpreted as low

    Urinalysis negative for hemoglobin

    Iron Studies

    Ferritin: 10

    TIBC: 350

    % Sat: 15

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    Diagnosis

    Colonoscopy revealed smallsuspicious lesion in sigmoidcolon, pathology revealingadenocarcinoma.Excisedsurgically, no mets.

    Routine labs, one year later,reveal an HCT of 40%. He feelsbetter than ever!

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    8.

    42 yo admitted with anemia

    Hemoglobin 8.8 g/dl

    MCV 80 fL Retic 5.8%

    WBC 12.0/uL

    86% PMN

    10% lymphs

    4% monocytes

    Platelets 676/uL

    ?

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    9.

    80 yo CM admitted for diarrhea, anorexia, fall

    PMH EtOH, hemicolectomy for CA

    Hgb 9.4 g/dL LDH 600 U/L

    MCV 124 fL WBC 3.4

    Plt 144

    Retic 1.4%

    ?

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    10.

    26 yo CM.

    Hct 36 Meds: none

    WBC 5.6 PMH: none

    Plt 214

    LDH nl Hapto

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    11.

    36 yo AAM with fever, rash, arthralgias, pain

    PMH: SS dz

    Hgb 5.2 LDH 612

    MCV 88 Bili 4.5

    WBC 5.0 plt 130

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    12.

    51 YO female presents with fatigue, occasional tingling of her hand and

    feet. She reports decrease in concentration and memory

    PSHx: cholecystectomy, gastric bypass

    Social Hx: negative for drug, tobacco and alcohol

    LABS:WBC 1.7

    HGB 8.9 G/DL

    PLATELETS 109,000

    MCV 109

    SEGS 52%

    LYMPHS 40%

    MONO 5%

    EOS 2%

    METAMYELOCYTES 1%

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    13.

    47 YO African American Female presents with fatigue, heavy menstrualbleeding, body aches.

    FHx: anemia of unknown etiology

    Social Hx, PMHx is unremarkable

    WBC 5K, HGB 9.8 g, PLT 166,000, MCV 56

    How do you approach this case?

    Serum Ferritin 15

    Iron saturation 9%

    TIBC 470

    B12 and folate are normal

    Retic 2.6%

    Bone marrow biopsy ?